抗中性粒细胞胞质抗体相关性间质性肾炎

49岁女性,主要表现为乏力、贫血、肾功能不全,无蛋白尿、血尿,无咯血等肺部表现,血核周型抗中性粒细胞胞质抗体(pANCA)阳性,弹性蛋白酶ANCA阳性。肾活检组织学表现为间质性肾炎,伴肾小球废弃,非废弃肾小球病变轻微。免疫病理示寡免疫复合物沉积,经甲泼尼龙和环磷酰胺治疗后,血ANCA转阴,重复肾活检肾小管间质急性病变亦明显减轻,肾功能明显改善,综合临床、实验室检查及组织学特点,最终诊断为ANCA相关性间质性肾炎。     

抗中性粒细胞胞质抗体阳性的感染性心内膜炎18例临床分析

目的:通过分析ANCA阳性的感染性心内膜炎（IE）的临床特点,旨在提高临床诊疗水平。方法:收集2016年6月至2021年7月在郑州大学第一附属医院住院确诊为IE且同时伴有血清学ANCA阳性的18例患者的临床资料,回顾性分析其一般情况、临床表现、实验室检查、影像学检查、治疗及转归。采用SPSS 20.0软件进行统计分析。...     

老年抗中性粒细胞胞质抗体相关性血管炎12例

<正>抗中性粒细胞胞质抗体(ANCA)相关性血管炎是一组累及全身多系统的自身免疫性疾病,因其临床表现不典型,并且常伴有多系统的损害,容易导致误诊误治〔1〕。本文分析我院12例老年ANCA相关性血管炎患者,更深入认识ANCA相关性血管炎所致全身多系统损害,从而早期诊断与治疗。1资料与方法1.1对象1999年10月至2012年12月12例老年ANCA相     

伴抗中性粒细胞胞质抗体阳性的抗肾小球基膜肾炎患者临床病理特征及预后

目的：探讨伴抗中性粒细胞胞质抗体(ANCA)阳性的抗肾小球基膜(GBM)肾炎患者临床、病理特征及预后。方法：选取2004年1月至2020年12月在国家肾脏疾病临床医学研究中心住院治疗的抗GBM肾炎患者,分析伴ANCA阳性患者的临床病理特征及预后,并与ANCA阴性患者进行比较。结果：192例抗GBM肾炎患者中,33例(17%)合并血清ANCA阳性,均为髓过氧化物酶(MPO)ANCA,其中2例同时合并蛋白酶3(PR3)ANCA阳性。与ANCA阴性组相比,ANCA阳性组患者发病年龄偏高、女性占比多、血清白蛋白水平高(P<0.05),而24 h尿蛋白定量、抗GBM抗体滴度低于对照组(P<0.05),病理上除肾小管间质急性病变较轻(P=0.027),两组无明显差异。ANCA阳性组患者总体预后较差,随访1年两组的人、肾存活率均无统计学意义。结论：伴ANCA阳性的抗GBM肾炎患者临床和病理特征与不伴ANCA的抗GBM肾炎相似,预后较差。     

狼疮肾炎抗中性粒细胞胞质抗体检测的临床意义

抗中性粒细胞胞质抗体 (ａｎｔｉｎｅｕｔｒｏｐｈｉｌｃｙｔｏｐｌａｓｍｉｃａｎｔｉ ｂｏｄｉｅｓ ,ＡＮＣＡ)是以中性粒细胞和单核细胞胞质成分为靶抗原的自身抗体 ,是血管炎的血清学标志之一 ,可根据细胞间接免疫荧光染色部位的不同分为核周型 (Ｐ ＡＮＣＡ)和胞质型 (Ｃ ＡＮＣＡ)。文献 [1,2 ]认为Ｃ ＡＮＣＡ对Ｗｅｇｅｎｅｒ肉芽肿的诊断有较高特异性 ,而Ｐ ＡＮＣＡ常与肾脏损害的血管炎有关。其在狼疮肾炎 (ＬＮ)发病机制中的作用 ,国外有较多的研究 ,国内仅少量、较少病例的报道 ,且结论不一。为此我们对 172例ＬＮ患者进行了Ａ…     

抗中性粒细胞胞质抗体相关小血管炎误诊17例临床分析

报道误诊为其他疾病的17例抗中性粒细胞胞质抗体（ANCA）相关小血管炎（AASV）患者,其误诊原因主要为临床医师对AASV缺乏认识及警惕,没有详细系统地询问病史及既往史,患者首发表现缺乏特异性。常规开展ANCA、抗髓过氧化物酶抗体和抗蛋白酶3抗体检测,提高对AASV的认识及警惕性,有助于AASV的诊断。     

抗中性粒细胞胞质抗体相关性肾炎患者的妊娠结局

目的:通过对5例抗中性粒细胞胞质抗体(ANCA)相关肾炎(AAGN)患者共7次妊娠的随访,探讨AAGN患者的妊娠合并症和胎儿结局及其影响因素.方法:5例抗髓过氧化酶(MPO)-ANCA阳性AAGN女性患者,在获得肾脏缓解后妊娠.孕期给予激素或联合硫唑嘌呤维持,血管炎活动性采用伯明翰血管炎活动评分(BVAS)评分.患者妊...     

抗中性粒细胞胞质抗体相关性肾炎诊治进展

摘要 抗中性粒细胞胞质抗体（ANCA）相关性血管炎（AAV）常出现肾脏受累，称为ANCA相关性肾炎（AAGN）。AAGN对患者的生存和长期预后有重要影响，早期诊断和及时适当的治疗至关重要。近年来，国内外大量临床试验的开展以及多部指南的发布为AAGN患者的临床管理决策提供了重要依据和宝贵指导意见。本文就此对AAGN的诊治进展进行简要概述。     

丙基硫氧嘧啶致抗中性粒细胞胞质抗体阳性小血管炎二例

丙基硫氧嘧啶 (PTU)是硫脲类抗甲状腺药 ,是临床最常用的抗甲状腺功能亢进药物之一。抗中性粒细胞胞质抗体(ANCA)是原发性小血管炎的血清学诊断工具。近年来发现有少数服用PTU的甲状腺功能亢进患者临床上出现了小血管炎的表现 ,并且在这些患者血清中也可检测到ANCA。现将我院近年发现的 2例PTU相关的ANCA阳性小血管炎报道如下。例 1 :女性 ,6 0岁。因皮疹、关节痛半年 ,泡沫尿 1个月入院。患者 3年前查血小板减低 ,骨髓细胞学示“巨核细胞增多伴成熟障碍” ,查自身抗体均阴性 ,临床诊断为特发性血小板减少性紫癜 (ITP)。予环孢菌…     

抗中性粒细胞胞质抗体的检出率及其靶抗原研究

目的　了解抗中性粒细胞胞质抗体 (ANCA)阳性检出率、流行病学特点及其靶抗原。方法　应用间接免疫荧光法 (IIF)、抗髓过氧化物酶 (MPO)和抗蛋白酶 3(PR3)酶联免疫吸附试验(ELISA)对近年送检的怀疑小血管炎的 5 6 0 4例患者血清进行了检测 ,对IIF ANCA阳性而抗MPO和抗PR3抗体均阴性的血清还进行了其他 5种ANCA特异性靶抗原的检测。并初步对ANCA阳性患者流行病学特点进行分析。结果　IIF ANCA检出率为 5 3% ,阳性检出最多在 7、8及 12月份。另外所有血清中有 390例 (7% )ANA阳性。所有血清进行抗MPO和抗PR3 ELISA检测 ,抗MPO抗体阳性 2 13例 ,抗PR3抗体阳性 32例 ,两者同时阳性 5例。 4 8例不识别MPO和PR3而IIF法阳性的血清中 13例识别其他已知靶抗原 ,识别杀菌 /通透性增高蛋白 (BPI)、人弹力蛋白酶 (HLE)、组蛋白酶G (CG)、天青杀素 (AZU)和乳铁蛋白 (LF)等靶抗原的血清分别为 7、5、1、1、0例 ,其中 1例为抗BPI和抗HLE ANCA同时阳性。 85 %的IIF ANCA阳性患者确诊为ANCA相关小血管炎。这些患者中 ,抗MPO和抗PR3的比例约为 7∶1:男女比例为 1∶1 12 ,年龄 7～ 79岁 ,平均 5 3 1岁 ,>6 0岁的老年人男女比例为 1 17∶1,而年龄 <2 0岁患者男女比例为 1∶4。结论　ANCA相关疾病在我国并不少见 ,以IIF法检     

原发系统性抗中性粒细胞胞质抗体阴性小血管炎的临床病理表现

目的总结分析原发系统性小血管炎(PSV)抗中性粒细胞胞质抗体(ANCA)阴性小血管炎患者的临床病理特点,并与ANCA阳性患者进行比较,以提高对该类疾病的认识。方法回顾性总结并分析我科近7年来确诊的13例原发系统性ANCA阴性小血管炎的临床病理资料,并与同期30例ANCA阳性原发性小血管炎患者进行比较。结果ANCA阴性小血管炎组患者13例,占同期诊断的原发性小血管炎患者的7.14%。与ANCA阳性组相比,阴性组皮肤和消化道受损比率显著增高,而发热及中重度贫血发生率低于阳性组,阴性组蛋白尿水平高于阳性组,两组血管炎活动积分无差异。阴性组无一例血免疫球蛋白G升高,与阳性组相比差异有统计学意义(0vs50%,P<0.01)。阴性组肾脏新月体形成发生率低于阳性组,坏死和间质小管病变两组差异无统计学意义。两组免疫抑制治疗方案和临床缓解率差异无统计学意义。结论原发性ANCA阴性小血管炎并不罕见,毛细血管襻纤维素样坏死和无免疫球蛋白G升高有助于诊断。     

多发性肌炎/皮肌炎合并肺间质病变的临床特征和预后

目的 研究多发性肌炎(PM)和皮肌炎(DM)合并肺间质病变(ILD)的临床特点和预后.方法 回顾性分析107例PM/DM患者的临床资料,包括首发症状、临床表现、实验室检查、影像学资料、治疗及预后.结果 107例PM/DM患者合并ILD有28例,ILD发生率为26.2%.①合并ILD的首发症状为关节炎或关节痛者高于无ILD(P<0.05);合并ILD临床表现为关节炎或关节痛、发热、干咳气促者明显高于无ILD(P<0.05).②DM合并ILD患者大多有特异性皮疹,呼吸困难较重(P<0.05),而PM合并ILD患者肌痛、肌无力较重(P<0.05).③合并ILD的红细胞沉降率(ESR)和C反应蛋白(CRP)明显高于无ILD(P<0.05);DM-ILD组肌酶谱以羟丁酸脱氢酶(HBDH)、天冬氨酸转氨酶(AST)升高为主(P≤0.05),PM合并ILD患者肌酶谱以肌酸激酶(CK)和肌酸激酶同工酶(CK-MB)为主(P<0.05).④合并ILD的28例PM/DM患者经治疗,20例病情改善,8例重症7例均为DM合并ILD,5例治疗无效因Ⅰ型呼吸衰竭死亡(病死率占PM/DM合并ILD患者的17.9%).结论 ①首发症状为关节炎或关节痛,临床表现有关节炎或者关节痛、发热以及ESR和CRP高者易合并ILD;有特异性皮疹、AST升高的DM易合并ILD;肌酶以CK和CK-MB升高为主的PM易合并ILD.②DM合并ILD病情进展凶险,病死率高,预后不良.     

Atypical, cytoplasmic and perinuclear anti-neutrophil cytoplasmic antibodies in patients with inflammatory bowel disease

Atypical, cytoplasmic and perinuclear anti-neutrophil cytoplasmic antibodies (x-, c- and pANCA, respectively) are associated with a variety of inflammatory diseases, including inflammatory bowel disease (IBD). Anti-neutrophil cytoplasmic antibodies are more common in patients with ulcerative colitis (UC) than in patients with Crohn's disease (CD). Most publications only refer to p- and cANCA in relation to IBD. We have prospectively evaluated the reactivity of sera from 58 patients with IBD and 10 healthy controls against human neutrophils with emphasis on the distinction of the ANCA types. The sera were incubated with ethanol- and formaldehyde-fixed granulocytes to differentiate between c-, p- and xANCA. The results showed that 10 of 24 patients with UC were positive for ANCA, whereas only one of 34 patients with CD was ANCA positive. These results correspond to a sensitivity of 42%, a specificity of 97%, a negative predictive value of 91% and a positive predictive value of 75% in UC. Of the 11 ANCA-positive sera, two showed a cytoplasmic staining pattern, three showed a perinuclear and six an atypical staining pattern. The disease activity was not correlated to either the ANCA titre or to the presence of ANCA in the serum. In conclusion, ANCA are of limited value in differentiating between UC and CD. Because the majority of ANCA in patients with IBD are xANCA, these ANCA should be explored by not only incubating on ethanol-fixed granulocytes, but also on formaldehyde-fixed granulocytes.     

以消化道出血为主要表现的抗中性粒细胞胞质抗体相关性血管炎临床分析

目的 分析以消化道出血为主要表现的抗中性粒细胞胞质抗体(ANCA)相关性血管炎患者的临床特征及预后.方法 查阅我院1987年5月至2007年5月收治的ANCA相关性血管炎合并消化道出血的病例.对患者生存率进行Kaplan-Meier统计分析.结果 ANCA相关性血管炎共170例,其中8例以消化道出血为主要表现,男性和女性各4例;平均年龄(39±20)岁;从疾病出现首发症状到消化道出血平均(8±8)个月;其中韦格纳肉芽肿病合并消化道出血最常见;临床表现最常见为便血(7例,占7/8),远端小肠为最常见受累部位(5例,占5/8);2例患者采用激素冲击治疗,6例采用相当于泼尼松1～2mg·kg-1·d-1激素治疗.1例患者行肠穿孔修补术,2例患者行病变肠段切除术,均是在出现外科情况后手术.3例存活,5例死亡,平均总存活时间为(373±108)d,出现消化道出血后患者平均存活时间为(46±10)d.结论 ANCA相关性血管炎合并消化道出血罕见,预后差.     

皮肌炎合并间质性肺疾病患者的临床特征分析

目的探讨皮肌炎(dermatomyositis,DM)合并间质性肺病(interstitial lung disease,ILD)患者的临床特点,以提高对DM-ILD的认识。方法搜集南京鼓楼医院自2006年6月至2013年6月期间临床确诊、资料完整的皮肌炎患者91例,对其中39例DM-ILD患者的临床特点及影像学检查进行分析。结果 91例患者中有间质性肺病者39例(42.8%),DM-ILD组发病年龄及病程与DM-无ILD组比较无明显差别(P0.05);DM-ILD组更常出现雷诺现象、关节痛、低热、咳嗽、咳痰及呼吸困难;DM-ILD组血沉(ESR)明显增快,C反应蛋白(CRP)明显增高,两组比较差异均有统计学意义(P0.01);抗核抗体(ANA)、乳酸脱氢酶(LDH)在两组间无明显差异,DM-ILD组抗Jo-1抗体阳性率增高;肺功能检查示DM-ILD组以限制性通气功能障碍及弥散功能障碍为主,DM-无ILD组肺功能正常;DM-ILD组胸部CT改变以磨玻璃影、条索状影及网格样改变最常见,胸腔积液少见;DM-ILD可增加死亡风险达2.3倍,感染是最常见的死亡原因。结论 ILD是DM常见的系统损害,DM-ILD的发生与疾病的活动及某些自身抗体有关,严重影响疾病预后,因此确诊DM的患者均应进一步明确肺部病变情况。     

Relationship between anti-neutrophil cytoplasmic antibody determined with conventional binding and the capture assay, and long-term clinical course in vasculitis

OBJECTIVES: To evaluate the relationship between anti-neutrophil cytoplasmic antibody (ANCA) measured with two different methods and long-term clinical course in vasculitis. DESIGN: Retrospective determination of ANCA with two different assays for detection of PR3-ANCA, conventional direct binding ELISA and capture ELISA using monoclonal antibodies against PR3. The 245 ANCA determinations were performed from frozen blood samples collected three to four times a year in each patient. SETTING: Department of Nephrology at a Swedish University Hospital. SUBJECTS: A total of 10 ANCA-positive patients with vasculitis caused by Wegener's granulomatosis (WG) or microscopic polyarteritis (MPA) and a very long follow-up time (mean 9 years, range 5-15.5 years). RESULTS: The total number of episodes with active vasculitis was 29 and all of them (100%) were detected by the capture technique whilst the conventional technique detected 23 (79%). The mean number of episodes with active disease requiring treatment with steroids and cytotoxic drugs was three per patient (range 1-6). At the time of clinical relapse of the vasculitis disease, the ANCA titre using the capture technique was either increasing or showed a very high value in all cases. The pattern of capture ANCA response could be subdivided into three categories: a close (four patients), an intermediate (three patients), and no (three patients) relationship between capture ANCA level and long-term clinical course. CONCLUSION: Detection of PR3-ANCA by the capture ELISA showed a higher sensitivity than that obtained by the direct ELISA in diagnosing relapse during follow-up of patients with vasculitis. The specificity of the capture ANCA was, however, low, as high levels occurred in patients without clinical disease activity.     

Nine patients with anti-neutrophil cytoplasmic antibody-positive vasculitis successfully treated with rituximab

OBJECTIVES: Rituximab (RIT) is a monoclonal anti-CD20 antibody, which depletes B-lymphocytes but not plasma cells. RIT is used for treatment of B-cell lymphomas, but has also shown beneficial effects in autoimmune diseases. In this case series RIT was used in anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis. DESIGN: Case series with a structured follow-up of treated patients. SETTING: Departments of Nephrology and Rheumatology of a university hospital. SUBJECTS: Two women with myeloperoxidase-ANCA-positive microscopic polyangiitis and seven patients (five men and two women) with proteinase 3-ANCA-positive Wegener's granulomatosis. All patients were resistant to conventional therapy or had relapsed repeatedly after cessation of cyclophosphamide (Cyc). INTERVENTIONS: The cases were treated with intravenous infusions of RIT once a week two times (three cases) or four times (six cases). To prevent formation of antibodies to RIT, mycophenolate mofetil (five patients), azathioprine (one patient), or a short course of Cyc (two patients) were added or allowed to continue. MAIN OUTCOME MEASURES: Remission at 6 months assessed with Birmingham vasculitis activity score. The cases were followed 6-24 months and relapse rate was also noted. RESULTS: Eight of nine patients responded completely and one case responded partially. Pulmonary X-ray improved (four cases), progress of lower extremity gangrene stopped (one case), remission of neuropathy was stable (one patient), renal vasculitis went into remission (two cases), and severe musculoskeletal pain improved (one case). Minor relapse in the nose occurred in two cases. No adverse events or major infections were noted. CONCLUSION: RIT seems promising and safe in ANCA-positive vasculitis, and controlled studies should be conducted.     

Analysis of clinical features and prognosis of anti-glomerular basement membrane antibody positive patients with anti-neutrophil cytoplasmic antibodies

正Objective To investigate the characteristics and outcome of glomerulonephritis in patients with both antineutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody.Methods The sera of 23 antiGBM glomerulonephritis patients were collected and were tested for ANCA respectively.Characteristics and outcome of patients with coexisting anti-GBM antibody and     

抗膜突蛋白抗体与系统性硬化病相关肺间质病变的临床相关性研究

目的 分析抗膜突蛋白抗体与系统性硬化病(SSc)相关肺间质病变(ILD)的临床相关性.方法 纳入参加欧洲抗风湿病联盟硬皮病试验研究组(EUSTAR)的SSc患者62例,采用酶联免疫吸附试验检测患者血清中的抗膜突蛋白抗体.分别根据高分辨CT特征、肺功能指标、炎症指标和病程的差异进行分组,比较不同分组之间抗膜突蛋白抗体的阳性率和滴度(吸光度值).计量资料采用独立样本t检验和非参数秩和检验,计数资料采用χ2检验.结果 SSc-ILD组的抗膜突蛋白抗体滴度(0.156±0.062)高于无ILD组(0.107±0.026),差异有统计学意义(P=005).在SSc患者中,检测抗膜突蛋白抗体对于诊断其合并ILD的敏感性为44.0%,特异性为91.7%(Kappa=0.2,P=0.022).肺高分辨CT上具有蜂窝样变、小叶间隔增宽及纵隔淋巴结肿大特征的SSc患者抗膜突蛋白抗体的阳性率显著高于不具上述特征者,差异均具有统计学意义(P<0.05).在肺总量(TLC%)减低组的患者中,该抗体的滴度(0.172±0.067)高于正常组(0.133±0.039).差异有统计学意义(P=0.011),一氧化碳弥散量(DLco%)减低组患者的抗体滴度(0.153±0.580)亦高于对照组(0.120±0.340),但差异无统计学意义(P=0.089).抗膜突蛋白抗体的阳性率在红细胞沉降率、C反应蛋白、免疫球蛋白和补体增高组与正常组间的差异无统计学意义(P>0.05).SSc病程≤5年组与>5年组的抗膜突蛋白抗体滴度差异无统计学意义(P=0.272),但ILD病程≤12个月者的抗体滴度(0.182±0.073)显著高于病程>12个月者(0.138±0.049),差异有统计学意义(P=0.040).结论 抗膜突蛋白抗体在SSc相关的ILD患者有较高的特异性,可能为揭示SSc-ILD的发病机制提供了新的线索.在SSc患者血清中检测该抗体对于早期筛查和评估ILD的价值值得进一步验证.